Cushing disease in pediatrics: an update.

Cushing disease (CD) is the main cause of endogenous Cushing syndrome (CS) and is produced by an adrenocorticotropic hormone (ACTH)-producing pituitary adenoma. Its relevance in pediatrics is due to the retardation of both growth and developmental processes because of hypercortisolism. In childhood, the main features of CS are facial changes, rapid or exaggerated weight gain, hirsutism, virilization, and acne. Endogenous hypercortisolism should be established after exogenous CS has been ruled out based on 24-hour urinary free cortisol, midnight serum or salivary cortisol, and dexamethasone suppression test; after that, ACTH dependence should be established. The diagnosis should be confirmed by pathology. The goal of treatment is to normalize cortisol level and reverse the signs and symptoms. Treatment options include surgery, medication, radiotherapy, or combined therapy. CD represents a challenge for physicians owing to its multiple associated conditions involving growth and pubertal development; thus, it is important to achieve an early diagnosis and treatment in order to control hypercortisolism and improve the prognosis. Its rarity in pediatric patients has led physicians to have limited experience in its management. The objective of this narrative review is to summarize the current knowledge about the pathophysiology, diagnosis, and treatment of CD in the pediatric population.

Association Between the Diabetes mellitus Duration and the Severity of Diabetic Foot Disease in Hospitalized Patients in Latin America.

The objective was to determine the association between a diabetes mellitus duration greater than 10 years and the severity of diabetic foot in hospitalized patients in Latin America.Analytical, observational, and retrospective study based in secondary databases. Patients older than 18 years with diagnosis of diabetes mellitus (DM) and hospitalized for any causes were included. The independent and dependent variables were having more than 10 years of diagnosis of DM and the severity of the diabetic foot disease (Wagner> = 2), respectively. A crude Poisson regression analysis was performed to obtain prevalence rates adjusted to confounders.Male gender was 54.8% and the median age was 62 years. In the group with more than10 years of disease (n = 903) 18% (n = 162) had severe injuries. We performed two Poisson regression analyzes, one of which included the entire sample; and in the other, only patients with some degree of ulcer were included at the time of evaluation (Wagner > = 1). In the first analysis the PR was 1.95 (p < 0.01) adjusted for the significant variables in the bivariate analysis and in the second analysis the PR was 1.18 (p < 0.01) adding to the adjustment the days of injury prior to hospitalization and the location of the ulcer.We conclude that in patients with more than 10 years of diabetes mellitus, diabetic foot injuries are more severe, regardless type of diabetes, gender, age, history of amputation and days of injury prior to hospitalization for inpatients in Latin America.

Complications of obesity in children and adolescents during covid-19 pandemic: A narrative review

Obesity in children and adolescents has increased exponentially around the world. Furthermore, the COVID-19 pandemic has led to a higher pediatric obesity rate. The excess adipose tissue generates a dysregulation of adiponectin, ghrelin, and leptin, among others. Metabolic alterations can develop cardiovascular disease, dyslipidemias, arterial hypertension, type 2 diabetes mellitus, nonalcoholic fatty liver disease, sleep disorders, and higher risk of COVID-19 severity. Obesity has different therapeutic approaches such as behavioral weight loss programs, pharmacologic treatments, and surgical procedures. Therefore, timely diagnosis and treatment are important to decrease the mortality in obesity among pediatric population.

La obesidad en niños y adolescentes ha aumentado exponencialmente en todo el mundo. Asimismo, la pandemia del coronavirus (COVID-19) ha conducido a una mayor tasa de obesidad pediátrica. El exceso de tejido adiposo genera una desregulación de hormonas como adiponectina, ghrelina y leptina, entre otras. Las alteraciones metabólicas pueden ocasionar enfermedad cardiovascular, dislipidemias, hipertensión arterial, diabetes mellitus tipo 2, enfermedad del hígado graso no alcohólico, trastornos del sueño y mayor riesgo de COVID-19 severo. La obesidad tiene diferentes enfoques terapéuticos, como programas conductuales de pérdida de peso, tratamientos farmacológicos y procedimientos quirúrgicos. De esta manera, el diagnóstico y el tratamiento oportuno es importante para disminuir la mortalidad asociada a la obesidad en la población pediátrica.

Late Diagnosis of Partial Androgen Insensitivity Syndrome in a Peruvian Child.

Disorders of sexual differentiation are congenital pathologies characterized by atypical development of genetic, gonadal, or phenotypic sex. These are caused by the alteration of any primordial phases of sexual development and may be evident at birth or in the later stage of life. Here, we present the case of a nine-year-old Peruvian school patient who has female gender assigned at birth, has no contributory antecedents and was found to have clitoromegaly and hypospadia on physical examination. In the blood tests, anti-Müllerian hormone and testosterone were found, and 46 XY karyotype and sex-determining region Y (SRY) genes were present. On abdominal ultrasound, testicles were found in the inguinal canals. The human chorionic gonadotropin (HCG) stimulation test was conducted, which allowed us to rule out defects in testosterone biosynthesis and enzyme defects in dihydrotestosterone production; the main suspected diagnosis was partial androgen insensitivity syndrome (PAIS). A multidisciplinary medical meeting was held, accepting the patient's desire to opt for the male gender, after acceptance by the parents. Thus, the patient underwent bilateral orchidopexy and genitoplasty. He is currently receiving therapy with testosterone, with an adequate response to the treatment and the molecular study confirmed the androgen-receptor gene mutation. In conclusion, we highlight the importance of a timely multidisciplinary diagnosis and management of disorders of sexual differentiation to avoid premature gender assignment and major social and family repercussions that it implies.

Familial Chylomicronemia Syndrome-Induced Acute Necrotizing Pancreatitis during Pregnancy.

Acute pancreatitis is a rare condition in pregnancy, associated with a high mortality rate. Hypertriglyceridemia represents its second most common cause. We present the case of a 38-year-old woman in the 24th week of gestation with a history of hypertriglyceridemia and recurrent episodes of pancreatitis. She was admitted to our hospital with acute pancreatitis due to severe hypertriglyceridemia. She was stabilized and treated with fibrates. Despite her favorable clinical course, she developed a second episode of acute pancreatitis complicated by multi-organ dysfunction and pancreatic necrosis, requiring a necrosectomy. The pregnancy was ended by cesarean section, after which three plasmapheresis sessions were performed. She is currently asymptomatic with stable triglyceride levels. Acute pancreatitis due to hypertriglyceridemia represents a diagnostic and therapeutic challenge in pregnant women, associated with serious maternal and fetal complications. When primary hypertriglyceridemia is suspected, such as familial chylomicronemia syndrome, the most important objective is preventing the onset of pancreatitis.